Aurocephalosyndactyly

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ORPHA:1219OMIM:109050
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8Treatment centers

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Overview

Aurocephalosyndactyly is an extremely rare genetic condition that belongs to a group of disorders known as acrocephalosyndactyly syndromes. These conditions involve an abnormal shape of the skull (craniosynostosis, where the bones of the skull fuse too early) combined with webbing or fusion of the fingers and/or toes (syndactyly). The name 'auro' refers to features that may also involve the ears. Because the skull bones close prematurely, the head may develop an unusual tall or pointed shape, and the brain may not have enough room to grow normally, which can sometimes lead to increased pressure inside the skull. Children born with this condition typically show visible differences in the shape of their head and hands at birth. The degree of finger and toe fusion can vary, and some children may also have differences in their facial features, such as widely spaced eyes or a flat midface. Hearing problems and developmental delays may also occur in some cases. Because aurocephalosyndactyly is so rare, there is no single standard treatment protocol. Management usually involves a team of specialists and may include surgery to release the fused skull bones (to allow normal brain growth), surgery to separate fused fingers (to improve hand function), and supportive therapies such as speech therapy and occupational therapy. Early intervention is important to give children the best chance for healthy development.

Also known as:

Key symptoms:

Abnormally shaped skull (tall or pointed head)Early fusion of skull bones (craniosynostosis)Webbed or fused fingersWebbed or fused toesFlat or underdeveloped midfaceWidely spaced eyesEar abnormalities or hearing problemsPossible developmental delaysPossible learning difficultiesDifficulty with fine motor skills due to fused fingersIncreased pressure inside the skull

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Aurocephalosyndactyly.

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No actively recruiting trials found for Aurocephalosyndactyly at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Aurocephalosyndactyly community →

No specialists are currently listed for Aurocephalosyndactyly.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Aurocephalosyndactyly.

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Community

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.How severe is the skull fusion, and when should surgery be done?,What is the plan for separating the fused fingers, and how many surgeries might be needed?,Should my child have genetic testing, and what will the results tell us?,What signs of increased pressure in the skull should I watch for at home?,Will my child need hearing or vision support?,What therapies should we start early to support development?,Is there a risk that future children could also have this condition?

Common questions about Aurocephalosyndactyly

What is Aurocephalosyndactyly?

Aurocephalosyndactyly is an extremely rare genetic condition that belongs to a group of disorders known as acrocephalosyndactyly syndromes. These conditions involve an abnormal shape of the skull (craniosynostosis, where the bones of the skull fuse too early) combined with webbing or fusion of the fingers and/or toes (syndactyly). The name 'auro' refers to features that may also involve the ears. Because the skull bones close prematurely, the head may develop an unusual tall or pointed shape, and the brain may not have enough room to grow normally, which can sometimes lead to increased pressur

How is Aurocephalosyndactyly inherited?

Aurocephalosyndactyly follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Aurocephalosyndactyly typically begin?

Typical onset of Aurocephalosyndactyly is neonatal. Age of onset can vary across affected individuals.